Clinical characteristics of patients with granulomatosis with polyangiitis and microscopic polyangiitis in ENT practice: a comparative analysis.

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Clinical characteristics of patients with granulomatosis with polyangiitis and microscopic polyangiitis in ENT practice: a comparative analysis.

Acta Otorhinolaryngol Ital. 2018 Dec;38(6):517-527

Authors: Wojciechowska J, KręCicki T

Abstract
ENT manifestations are commonly observed in patients with small vessel vasculitis (SVV). The main aim of this study was to analyse and present the clinicopathological characteristics of individuals with SVV emphasising otorhinolaryngological symptoms. This study evaluated 64 patients, 41 with granulomatosis with polyangiitis (GPA) and 23 with microscopic polyangiitis (MPA). Herein, we compare the clinicopathologic features of GPA and MPA. The average age at diagnosis was 50.2 and 56.2 years, for GPA and MPA, respectively. 57 patients (89%) were antineutrophil cytoplasmic antibody (ANCA) positive, 34 (59.6%) for anti-proteinase 3 (PR3)-ANCA and 21 (36.8%) for myeloperoxidase (MPO)-ANCA. 7 patients (10.9%) were ANCA negative. The most commonly affected organs were lungs (76.56%), ear, nose, throat (ENT) (75%) and kidneys (73.44%). ENT disorders mainly appeared as chronic rhinosinusitis and epistaxis and preceded SVV diagnosis by an average 14.4 months. In the majority of patients, ENT disorders were the first symptoms of SVV and preceded its systemic transformation. Pulmonary, ENT and nervous manifestations were more common in GPA, whereas the prevalence of renal, gastrointestinal, cutaneous, cardiovascular and ocular disorders was higher in MPA. The results of our study emphasise the high prevalence of ENT symptoms in patients with SVV, especially in those with GPA. We highlight the significant role of the otorhinolaryngologist in early SVV diagnosis and management. Any patient with persistent ENT symptoms or ENT dysfunctions not responding to standard otorhinolaryngological treatment should be precisely and rapidly evaluated for the presence of systemic dysfunctions (especially renal and pulmonary). Realising the differences and similarities between GPA and MPA is crucial in undelayed SVV diagnosis and proper treatment.

PMID: 30623897 [PubMed - in process]

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